Background: Small cell carcinoma of the ovary (SCCO), hypercalcemic type is a rare and often aggressive tumour, occurring mostly in young women. In tow third of reported cases, there is an associated paraneoplastic hypercalcemia. Up to present, there were described four cases of SCCO in child. This report gives a literature review, describe the histological and immunohistochemical features and discuss the differential diagnoses of this rare entity.
Case Reports: We report the fifth pediatric case in a 10-year-old girl. The patient underwent a salpingooophorectomy and was found to have stage IA disease. She received multiagent chemotherapy and was well 2 years after diagnosis.
Conclusions: Although rare, SCCO should be considered in the differential diagnosis of any ovarian neoplasm composed of round cells particularly in childhood.

Keywords: small cell carcinoma, Hypercalcemia, childhood cancer